Metastatic Crohn disease is a rare cutaneous disorder characterized by noncaseating, granulomatous skin lesions present at sites anatomically separate from the gastrointestinal tract. It is the least common dermatologic manifestation of Crohn disease, and the differential diagnosis includes numerous similarly appearing, granulomatous skin entities. Males and females appear to be equally affected, and children tend to present with clinical lesions different from those of adults. An integration of clinical information, microscopic findings, and ancillary studies is necessary to accurately diagnose this rare cutaneous disease. Our objective is to review the clinical features, histopathologic characteristics, suggested pathogenesis, differential diagnosis, and current therapeutic options of metastatic Crohn disease.